Pediatric Epilepsy Focues on Monitoring, Early Surgery

Established in 2007, the UCSF Pediatric Epilepsy Center offers comprehensive services to children with epilepsy, including high-quality imaging, psychological evaluations, pharmaceutical treatments and surgery. Although it provides care for all children with epilepsy, the center specializes in monitoring and treating the 10-to-20 percent of pediatric epilepsy cases that don't respond well to medication and so are considered intractable.

While pediatric epilepsy shares some similarities to adult epilepsy, fundamental differences exist. Epilepsy in children is often the result of aberrant development of the child's nervous system. This may be the result of either early malformations of cortical development or acquired processes such as perinatal stroke. When such insults happen early, the child's developing brain has the ability to adapt in a way that an adult's brain cannot. This cerebral plasticity allows the developing brain to reorganize important functions in other, unaffected areas, which offers exciting opportunities for treatment.

Taking a multidisciplinary approach to treating its young patients, the center utilizes specialists not only in neurology and epilepsy, but also in child life and neuropsychology. "Many children with epilepsy have other cognitive, affective or developmental issues," says Joseph Sullivan, M.D., director of the center. "It can be difficult to fully evaluate a child's epilepsy unless his other strengths and weaknesses have been identified."

Monitoring Capabilities

The center specializes in high-quality, extended video EEG monitoring, high-resolution 3-tesla MRI for structural imaging, magnetoencephalography (MEG) and functional MRI-based brain mapping. "In a minority of patients, an MRI shows a lesion on the brain that is causing the seizures," says Nalin Gupta, M.D., Ph.D., chief of UCSF Pediatric Neurological Surgery. "The majority of cases, however, are nonlesional, which means we have to try to locate the source of the seizures by mapping out electrical activity in the brain."

To bolster its monitoring capabilities, the Pediatric Epilepsy Center is currently constructing four video EEG monitoring rooms, which will house the latest video EEG equipment. These technologies help the center's physicians to more accurately identify the regions of the brain that are the seizures' focus.

Treatment Options

The center does use medications to treat its epilepsy patients, but Sullivan recognizes the "big debate" around that treatment option. "It's a tough balancing act," he says. "The side effects from seizure medications can be very profound. It can be hard to figure out when cognitive impairments or other side effects are the result of seizures or when they are the result of the medication."

The ultimate goal, he notes, "is for the child to have no seizures and no side effects. Some kids will say they can tolerate mild seizures as long as there are no side effects, but this is not the ultimate goal."

UCSF neurologists and neurosurgeons consider surgical options earlier than other specialists might. "If a child's seizures are well controlled with medication, we don't usually consider surgery as an initial step," says Gupta. "But if a child has tried one or two medications and the seizures are still happening, we suggest an evaluation for surgery. Children's brains are still developing. If seizures are hampering that development, we like to intervene earlier in hopes of getting a better outcome."

If one or two medications do not work, Sullivan adds, "there's a low likelihood of a third medication working. We know that in the past, doctors were worried about performing brain surgery on young patients. But we now believe that surgery may be the best option if pharmacological solutions have failed."

Currently, the center offers four types of surgery. Lesionectomies are performed when the MRI scan shows a clear abnormality in the brain tissue that is concordant with findings on scalp video EEG monitoring.

In cases where surface readings of seizure activity are inexact or there is a nonfocal MRI finding, patients undergo a two-step surgery that involves electrocorticography, or ECoG. In the first step, an operation is performed to open a window through the skull, and an array of electrodes known as a subdural grid is placed directly on the brain surface, so that brain activity can be monitored in the hospital over the course of several days.

In the second step, the portion of the brain that has been shown to be generating the seizure activity is removed. The two-step process allows surgeons to pinpoint epileptogenic tissue, and thus to spare healthy brain tissue while resecting the brain.

Patients suffering atonic seizures may also be candidates for corpus callosotomy, in which the surgeon cuts the corpus callosum to prevent seizures from spreading from one side of the brain to the other.

For children who are not good candidates for those surgeries, UCSF neurosurgeons can implant vagal nerve stimulators in the chest and connect them to the vagus nerve in the neck. The device is preprogrammed to deliver periodic "doses" of electrical currents to prevent seizures from occurring. If a seizure occurs between the currents, the patient (or a caregiver) can pass a magnet over the device to trigger an additional "dose."

All of the doctors at the center work closely with child life specialists, to be sure that each patient's experience at the center is as child-friendly as possible.

"If we're doing extended video and electroencephalogram monitoring, the children may end up staying here five to seven days, or sometimes even longer, during which time their EEG activity will be continuously monitored," says Sullivan.

"It is important for the EEG electrodes to be placed in such a way to ensure a high-quality recording," Sullivan explains. "Our child life specialists have developed distracting techniques for getting the EEG leads onto patients' heads, and do everything they can to help keep the children 'entertained' during their stay. At present, this involves participating in a number of activities in the EEG monitoring room, but we are currently developing a mobile EEG unit that would allow the kids to go to the playroom and still be monitored."

Social workers also are available to counsel the parents before brain surgeries are undertaken. "Most parents who have children with intractable epilepsy have watched their child cope with multiple seizures on a daily basis for years," Gupta says. "The child may be functional, but often there has been a profound impact on the family and on family dynamics. Reducing the number or severity of the child's seizures is a goal they want to achieve. They know it can greatly affect their child's quality of life."

Who should be referred?

The Pediatric Epilepsy Center welcomes all children with epilepsy, but recommends that physicians refer children who:

Are having seizures that are unresponsive to one or two medications

Are suffering intolerable side effects from medications

Are having repetitive "spells" where it is unclear whether they represent seizures

"We'd rather see these patients right away than have them wait three or four years before coming to us," notes Sullivan.

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